Article Text

PDF
AB0651 Eosinophilic Fasciitis (Schulman's Disease): Clinical Spectrum, Response to Treatment and Long Term Outcome in A Series of 18 Spanish Patients
  1. W.A. Sifuentes Giraldo1,
  2. V. Maldonado Romero1,
  3. I. Amil Casas1,
  4. C. Velásquez Arce1,
  5. C. Guillén Astete1,
  6. M. Vázquez Díaz1,
  7. J. Bachiller Corral1,
  8. C. de la Puente Bujidos1,
  9. E. de las Heras Alonso2,
  10. J.L. Calleja Lόpez3,
  11. J.L. Patier de la Peña3,
  12. M.Ά. Moreno Cobo3,
  13. A. Zea Mendoza1
  1. 1Rheumatology
  2. 2Dermatology
  3. 3Internal Medicine, University Hospital Ramon y Cajal, Madrid, Spain

Abstract

Background Eosinophilic fasciitis (EF) is a rare disorder characterized by symmetrical skin induration of the limbs, peripheral eosinophilia, and characteristic histopathological changes in the muscular fascia. Although it has been considered as a disorder limited to the skin and soft tissues, there is evidence of immunological abnormalities and visceral involvement in some patients.

Objectives To study the spectrum of clinical and immunological manifestations, response to treatment and long-term outcome of patients with EF diagnosed in a tertiary Spanish hospital.

Methods All the patients diagnosed with EF during the period 1983-2013 were included. Demographic, clinical, immunological, therapeutic and outcome data were obtained by reviewing the medical records and analyzed using the statistical software SPSS 21.0.

Results A total number of 18 patients were included, 61% males, with a mean age at diagnosis of 51 years (range: 28-76). The onset was acute in 33% and subacute in 67%, and mean delay in diagnosis was 8±6.3 months. There was a history of physical exertion in 33% and occupational exposure to chemicals in 17%. Fever and constitutional symptoms were present in 22% of cases. The most common cutaneous manifestation was induration (89%), followed by edema (67%), hyperpigmentation (56%), erythema (39%), sclerodactyly (22%), `groove” sign (22%), pruritus (17%) and “orange peel” (11%). There was overlap with other forms of localized scleroderma in 3 cases (linear scleroderma 2, generalized morphea 1). The most common extracutaneous manifestations were musculoskeletal (arthralgia 56%, joint contractures 44%, myalgia 33%, and muscle weakness 22%). Carpal tunnel syndrome was detected in 22%, esophageal dysmotility in 11%, autoimmune thyroiditis in 6% and mild ventilatory restriction in 6%. Up to 49% showed increased acute phase reactants and 78% eosinophilia. The most common immunological abnormalities were the presence of antinuclear (28%),anti-thyroperoxidase (6%), and anti-parietal cells (11%) antibodies, and polyclonal hypergammaglobulinemia (33%). The fascia biopsy, performed in 10 cases, was consistent with EF in all them. Glucocorticoids were administered in 89%, anti-H2 22%, colchicine 17%, hydroxychloroquine 33%, methotrexate 22% and azathioprine 17%. Response to glucocorticoids was partial in all the cases. Remission was achieved with the immunosuppressive drugs in 33%, partial response in 33% and failure in 11%. A refractory case responded to PUVA photochemotherapy. After a follow-up period of 16±9.2 years of 16 cases, none of them progressed to systemic sclerosis, but 1 patient developed recurrent eosinophilic pneumonia and 1 died of a ruptured aortic aneurysm.

Conclusions Extracutaneous manifestations are common in EF, especially musculoskeletal, and a small proportion of patients may develop visceral involvement, including the digestive and respiratory systems. The response to steroid therapy is not optimal in all the cases and frequently require immunosuppressive drugs. However, long-term prognosis is usually favorable.

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.5570

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.